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Spastic disorder in patients with hereditary multiple exostoses, but without spinal cord compression: a new syndrome?

We describe a 37 year old man with a history of a gait disorder which had been worsening over a period of three years. Clinical examination showed the typical signs of a spastic tetraparesis with increased tone of all the extremities. Sensation, autonomic and cerebellar functions were not disturbed. Multiple exostoses had been present since early childhood, but none had been found in the spine or the cranium to cause the tetraspastic disorder. MRI scan was normal. Pedigree analysis of four generations showed that other family members were affected by both disorders. Chromosomal analysis was normal. We consider this to be a previously unknown hereditary syndrome transmitted as an autosomal dominant and manifesting a combination of spastic tetraparesis and multiple exostoses.

Adult↗

Effects of beraprost sodium on canine cauda equina function and blood flow using a chronic spinal cord compression model.

Changes in blood flow after chronic compression were observed in 19 dogs after 10 mmHg compression for 1 week before and 1 hour after the intravenous administration of one of three doses of beraprost sodium (BPS; 30 ng x kg(-1) x min(-1), n = 7; 100 ng x kg(-1) x min(-1), n = 7; and 300 ng x kg(-1) x min(-1), n = 5). The speed of blood flow was calculated using a specially designed microscope equipped with a video camera. Dogs treated with BPS had lesser degrees of reduction in their nerve conduction velocity compared with controls. A vascular mechanism of injury likely explains why BPS-treated dogs had a lesser degree of reduction in their nerve conduction velocities compared with the control population.

Animals↗

High dose versus low dose dexamethasone in experimental epidural spinal cord compression.

A compound of methylcellulose-silicone expanding progressively over a 1-week period by moisture absorption was implanted in the midthoracic epidural space of 17 Sprague Dawley adult rats. When the animals became paraplegic 6.3 +/- 1.6 days later, they were randomized into three groups: untreated control (n = 5), high dose dexamethasone (HD, 1.25 mg/kg intramuscularly, twice daily, n = 5), and low dose dexamethasone (LD, 0.125 mg/kg intramuscularly, twice daily, n = 7). Motor strength was evaluated daily by an observer unaware of the treatment given to the rats. Animals treated with dexamethasone (HD or LD) improved faster than untreated control animals. No significant difference in the rate of recovery or degree of motor improvement was noted between the HD and LD groups. Mortality was higher in the HD group because of infections and gastrointestinal perforation/bleeding.

Animals↗

Effective diagnosis of spinal cord compression using telemedicine.

We report the case of a man with an acute cervical cord lesion which was diagnosed after a teleconsultation with a neurologist at a distant neurological centre. We show that the ability to assess patients clinically using real-time videolinks is also of use in detecting those patients who would benefit from specialist neurosurgical intervention, but who might otherwise be denied it because of their location.

Acute Disease↗

Spinal cord compression by primary non-Hodgkin's lymphoma.

Epidural Cord Compression (ECC) by primary lymphomas is rare entity and constitutes less than 3% of total malignant lymphoma with Non-Hodgkin's Lymphoma (NHL), diffuse large cell type being the most common histological subtype. In this paper 16 cases of primary NHL with cord compression seen at the Department of Medical Oncology, during the period 1988-1990 are reviewed. At presentation all patients had undergone Laminectomy with decompression of epidural mass. The histological diagnosis of NHL was subclassified according to the International working formulation and was evaluated for disease process elsewhere in the body. All patients with ECC by lymphoma received high dose steroids with concurrent Radiotherapy (local) and combination Chemotherapy. These patients had longer duration of neurological deficit prior to treatment had poor response. After 6 courses of chemotherapy 50% of the patients had complete neurological recovery (CR), 31% had partial neurological recovery (PR) and in 19% there was no neurological recovery (NR).

Adolescent↗

[Epidural non-Hodgkin's lymphoma presenting as a spinal cord compression].

OBJECTIVE AND METHODS: epidural localization is a rare presenting sign of non Hodgkin's lymphoma. In this study, we describe the clinical, histological and immunohistochemical data in 13 cases (9 men and 4 women) of non-Hodgkin's lymphoma with epidural involvement. RESULTS: the median age was 63 years (range 36-76 years). Clinically, most patients complained of back pain (median duration, 3.4 months) followed by acute neurological deterioration. In 10 out of 13 tumors, a thoracic localization was observed and a decompressive laminectomy was performed in all cases. Histology and immunohistochemistry showed all tumors to be B-cell lymphomas which were classified as: lymphocytic lymphoma (3 cases), prolymphocytic lymphoma (1 case), follicular lymphoma (2 cases), diffuse large cell lymphoma (6 cases) and AIDS associated Burkitt lymphoma (1 case). Post-operative staging revealed bone and/or paravertebral involvement in 11 cases and disseminated disease in 5 cases. Median survival following complementary therapy, radiotherapy and chemotherapy in most cases, was 20 months. CONCLUSION: histological features of epidural lymphomas are similar to other extranodal lymphomas and their prognosis is better than other epidural neoplasms, especially when the tumor is localized.

Adult↗

Thoracic disc herniation and spinal cord compression. MRI and gadolinium-enhancement.

The authors present a study of 6 patients aimed at assessing the role of Magnetic Resonance Imaging with gadolinium-enhancement in the diagnosis of thoracic disc herniation. The results of MRI were compared with the findings of computed tomography, myelography and surgery. In two patients, the signal from the herniated disc was so low on all sequences that thoracic disc herniation was diagnosed only on the mass effect on the cord. The anterior longitudinal epidural venous plexus (AEVP) is displaced posteriorly and thickened in case of thoracic disc herniation. Gadolinium-enhancement of AEVP was marked in these conditions, giving a "tent-like" round configuration, or "lifted band" appearance. The exact volume and mass effect of the thoracic disc herniation were assessed by T1-enhanced images on the sagittal and axial planes.

Adult↗

Comparison of nuclear magnetic resonance imaging and computed tomography in the diagnosis of upper cervical spinal cord compression.

Because of its ability to visualize soft-tissue structures in greater detail than any previous radiographic modality, nuclear magnetic resonance (NMR) has been heralded as one of the greatest advances in recent years. It is particularly well suited in diagnosis of spinal disorders since it can visualize the spinal cord and cerebrospinal fluid without intrathecal contrast agents. This is a preliminary report of our experience of NMR examination in 106 patients studied since November 1982 at Case Western Reserve University Hospital. One of the best applications of NMR is for compressive lesions of the upper cervical spine because "functional" images can be obtained. The information from NMR was compared with computed tomography (CT) in 14 patients with various etiologies of neural compression--atlanto-occipital subluxation in Down's syndrome, fixed post-traumatic atlantoaxial subluxation, rheumatoid atlantoaxial subluxation and basilar impression of the odontoid, ossification of the posterior longitudinal ligament, Klippel-Feil syndrome, congenital spinal stenosis, cervical spondylosis, Arnold-Chiari malformation, etc. Unlike CT, NMR can directly image in the sagittal plane without computer reformatting of axial cuts. This readily provides direct images of the spinal cord in neutral, flexion, and extension to show the mechanism of neural compression. Disadvantages of NMR compared with CT include poorer image resolution, slightly increased imaging time, and no patients with intracranial aneurysm clips or cardiac pacemakers can be studied. Advantages include no radiation exposure, no intrathecal contrast agent is required to differentiate CSF from neural tissue, and direct sagittal, coronal, and oblique images can be obtained. As a result of this preliminary experience, computed tomography and nuclear magnetic resonance imaging appear to be complimentary studies--CT provides better osseous detail, whereas NMR offers superior soft tissue resolution.

Adult↗

Emergency magnetic resonance imaging of cervical spinal cord injuries: clinical correlation and prognosis.

OBJECTIVE: The goal of this study was to determine the prognostic and clinical value of magnetic resonance imaging (MRI) performed within hours after cervical spinal cord injuries in human patients. METHODS: Fifty-five patients with acute cervical vertebral column and spinal cord injuries underwent MRI as part of their initial treatment at the University of Michigan Medical Center. All images were obtained within 21 hours after injury (mean, 7.8 h) and were interpreted by an attending neuroradiologist who was blinded to the clinical status of the patients. Neurological function at presentation and in long-term follow-up examinations was compared with MRI characteristics assessed immediately after the injury. RESULTS: The presence and rostrocaudal length of intra-axial hematoma, the rostrocaudal length of spinal cord edema, the presence of spinal cord compression, and spinal cord compression by extra-axial hematoma were each significantly associated with poor neurological function at presentation and in long-term follow-up examinations. Although the best single predictor of long-term improvement in neurological function was the neurological function at presentation, four MRI characteristics, i.e., the presence of intra-axial hematoma, the extent of spinal cord hematoma, the extent of spinal cord edema, and spinal cord compression by extra-axial hematoma, provided significant additional prognostic information. MRI data demonstrated spinal cord compression for 27 of 55 patients (49%), leading to emergency surgery. Among patients who underwent imaging after restoration of normal vertebral alignment using closed cervical traction, 13 of 26 (50%) underwent emergency surgery for treatment of persistent, MRI-demonstrated, spinal cord compression. CONCLUSION: Emergency MRI after spinal cord injury provides accurate prognostic information regarding neurological function and aids in the diagnosis and treatment of persistent spinal cord compression after vertebral realignment.

Adolescent↗

[Compression of the spinal cord by the amyloid tissue in the thoracic vertebral amyloidoma].

Solitary amyloidoma consists of amyloid tissue deposits in the absence of systemic amyloidosis or plasma cell dyscrasia. Single vertebral amyloidoma is an unusual cause of spinal cord compression. We present the case of a 72-year-old man with medullar compression by amyloidoma affecting the sixth dorsal vertebra and with nuclear magnetic resonance images that were indistinguishable from epidural metastasis. The patient improved notably after resection.

Aged↗